New concepts of the etiology, diagnosis, and treatment of congenital megacolon .(Pediatrics, 1949; 4: 201-209

The clinical syndrome known as Hirschsprung disease (HD), or congenital megacolon, had been recognized for more than a century. The pathogenesis of the dilation/hypertrophy of the colon without mechanical obstruction was puzzling, and the subject of great debate. Virtually all infants and children affected with this disorder died. The authors studied 26 patients with congenital megacolon admitted to Boston Children's Hospital during a 5-year period, from 1943 through 1947. Careful contrast radiographic study of the distal colon in all 26 revealed a functional obstruction produced by a dyskinesia of the rectum. This was detected by running a small amount of barium slowly into the rectum with the patient in an oblique position. A portion of the rectum/rectosigmoid was consistently subnormal in caliber. Barium then was allowed to run through this narrowed area into the dilated colon. The authors interpreted the radiographic findings as evidence of a physiologic partial obstruction of the rectum/rectosigmoid. Colostomies were performed in the dilated portion of the colon in 3 early patients, and all symptoms/signs of HD disappeared in each instance. These returned, however, with closure of the colostomies and persisted until the ostomy was reestablished. Colostomies then were performed in an additional 12 patients, and each was relieved of symptoms with restoration of a normal-caliber colon. The authors recognized that the distal obstructing segment would have to be removed before the colostomy could be closed. They devised a special "pull-through" form of resection and tested it in the laboratory. The operation was performed subsequently on 23 patients, with no fatalities. Closure of the colostomy was possible in all but 2. The authors outlined the operation in detail in the article. Three infants, the youngest being 2 months old, tolerated a one-stage operation. The authors indicated that all patients subsequently demonstrated the urge to defecate and had normal bowel control. Patients with HD have a physiologic obstruction in the nondilated segment of colon. Early colostomy relieves the symptoms/signs of obstruction, and removal of the narrowed segment results in cure.